Disorder or Disease?

So here’s part 2 of our discussion from last week about whether VWD should be von Willebrand disorder or disease. We’ve gotten responses to our social media posts almost exclusively in favor of disorder!

But my former co-author, the late Renée Paper, had a point to make in 2002 when we first considered making a ruckus to change the name legally. In the first edition of our book, A Guide to Living with von Willebrand Disease, she wrote:

“For a number of years, I have heard from various people in the bleeding disorders community that the name von Willebrand disease (VWD) should be changed to von Willebrand disorder. I hear the same arguments that Laurie has made. Disease is a scary word, disorder is not. The word disease has a negative effect on a child’s self-esteem. Insurance companies are more likely to discriminate against someone with a disease rather than a disorder. Hemophilia is called a blood disorder, why isn’t VWD?

“As an emergency room nurse, I agree that parents have influence over their children. I also think that parents influence their children more with their attitudes than with their words. I believe that a child will accept the VWD diagnosis in a manner similar to that of the parent. If the parent accepts the

diagnosis and does not make a big deal over it, so will the child. If the parent has an intense negative reaction, it is likely that the child will react the same way. I believe that insurance companies do not care what it is called. The name of the disease is less likely to affect payment or coverage decisions than the symptoms and cost of care. Whatever it is called, von Willebrand disease, von

Willebrand disorder, von Willebrand syndrome, or von Willebrand’s, the symptoms are exactly the same and so is the cost for treatment.

“If VWD were new on the scene today and I had to name it, I would call it a disorder. But VWD was named about 70 years ago, and since then it has appeared everywhere as disease. Do we really need to draw this fine line now?

“The most important argument against changing the name is that VWD affects millions of people worldwide, yet it is grossly underrecognized and underdiagnosed. We need to devote all our energy and resources to educating others about the disease and its symptoms and to providing appropriate

laboratory evaluation and treatment. A name change would involve a vast amount of money and time. New educational materials would have to be printed, and the FDA would need to approve new labeling on medicines to treat it. It is no simple task to change the name.

“Around the world, medical literature universally calls it von Willebrand disease or VWD. For consistency, we should, too. Let’s not muddy the waters now when we are working so hard to spread the word and increase awareness. Money would be better spent by focusing on the big picture, that is, ending the suffering and isolation felt by the many people worldwide affected by VWD.

Renée Paper, RN

February 2002

So, what do you all think?  The Irish Haemophilia Society has decided to call it a disorder, because its members have requested it. Is it time for us too, to update the name to disorder?

You Say Tomato… I say Disorder

What’s in a Name?

I recently saw on Twitter that the Irish Haemophilia Society has decided to use the term disorder when referring to von Willebrand disease. It will now be called von Willebrand disorder. I want to shout hooray! At long last, one organization has seen the light!

Why at long last? Because I’ve been promoting this idea since 2002, when I published the world’s first book on VWD. The excerpt below is from that book, where my co-author Renée Paper and I published our differing opinions on the subject. And not too long ago, a debate erupted on Facebook about what to call VWD. Very heated, very polarized. Tonight I’ll share what I wrote back in 2002. Next week, I’ll publish what Renée, a nurse with VWD, believed.

Excerpted and Adapted from A Guide to Living with to Willebrand Disease, 2002:

For a while, my co-author Renée Paper and I weren’t sure what to title the world’s first book on VWD, published in 2002. We could not agree on what to call VWD—von Willebrand disease, von Willebrand, or break all the rules and call it von Willebrand disorder?

Co-authors Renée Paper, RN, and Laurie Kelley, 2002

I considered the question as a parent and a person with a degree in child psychology, who had published research on children’s concepts of health and illness in 1979. I questioned using “disease.” Is this what we really want to tell a child? It has long baffled me why the bleeding disorder community refers to hemophilia as a blood disorder but von Willebrand as a disease. Aren’t they both disorders? Can so simple a distinction have an impact on a person’s emotions, thoughts, and even health? In psychology, the meaning that is attached to words matters, especially to a child. I worry for the child who has VWD. To a child, the labels that a parent uses matter a great deal.

To people who work in the medical field, the term disease may not mean anything different than the term disorder. But to a child, disease may carry significant associations that are almost always negative. To a child, disease is usually akin to death—it can mean fatal, degenerative, and contagious.

Think of the impact of telling a child that he or she has von Willebrand disease. How does she view her condition in relation to others? Does he think that he could die of it? Will it get worse? Can others catch it? Calling it a disease, when it could be called a disorder, may provoke unnecessary anxiety, fear, and misunderstanding.

So why don’t we just change the name? I have been told that it’s more important for VWD to remain a disease for now. The bleeding disorder community needs healthcare dollars to educate the public and find those with VWD who remain undiagnosed and suffer in silence. So the community

continues to use a word that generates concern and commands attention. The word disease has impact.

In the end, Renée and I opted to use the conventional term to avoid rocking the political and medical establishments. But as a parent or as a person with VWD, you do not need to follow a political agenda with your child or your family. If you are the parent of a child with VWD, ask your child the difference between disease and disorder and listen what he or she thinks. When you explain diagnosis or treatment to your child, you may want to leave out the word disease and instead say von Willebrand or VWD. Ask your child’s doctors and nurses to do the same. More important than any political agenda and more important than what the medical books say is your child’s self-esteem. What you tell your child about VWD matters a great deal. Be sure it is as positive and empowering as possible.

Next week: Renée’s take on disease vs disorder.

Leadership, Remembered

This has been a week of leadership reflection regardless of who you voted for. I read from one news source that Biden made this a campaign about character; and many friends on Facebook stand by Trump for his policies. These traits, and so many others, are what make up leadership. While it has been a rocky week, and an exhausting, challenging year, it is all about leadership.

Laurie Kelley and Renee Paper 2002

I reflected on leadership this week coincidentally, while remembering one of the greatest and most unique leaders we ever had in the bleeding disorder community—Renée Paper. She passed away November 7, 2007, at age 49. If you ever got to meet her and hear her speak, you probably never forgot it. I always say she was a great lady, in size, intellect and passion. The most intelligent woman I ever met. She had a photographic memory, razor sharp tongue, ribald sense of humor, love of animals and fiery passion to help people. She was an emergency room nurse who had von Willebrand disease (VWD), and truly became our foremost spokesperson and advocate for women with VWD. After hearing her speak (like a cannon going off) at various events five times, I was so excited myself (why wasn’t anyone doing anything to help those with VWD?), that I talked her into co-authoring a book with me, which became the world’s first book on VWD, published in 2004.

I could not imagine that I’d lose this amazing person, who lectured like an old time preacher, who could draw audiences from young women to seasoned hematologists, who pounded the podium in her quest to get the NHF, the country, doctors, patients and even the government to take women with bleeding disorders seriously. Who can forget her favorite line? “Know why doctors don’t take us seriously when we tell them we bleed too much and too often? Because men don’t have uteri!” And she would smile to see the audience either squirm (the men) or roar with laughter and approval (the women).

She and I traveled together, presented together, wrote together. She loved coming to visit my zany household (“You let your kids roller skate in the house?” she asked incredulously) And then proceeded to reprimand me for not giving my elderly dog pain killers.

I loved Renée and sadly witnessed her demise; years of health issues took their toll. She died far too young. She had character and was a character. She also possessed those vital traits of great leaders: compassion, boundless energy, drive, passion, and vision. She didn’t live long enough to see her vision come true. We do have much better care for those with VWD now. But I often wonder hw much further we could have gone, had this amazing leader stayed with us a bit longer.

A new edition of A Guide to Living with von Willebrand Disease will be available in 2021.

A Star is Born

Continued from last week; a profile in leadership. Andrea Trinidad, woman with VWD, who is changing lives for all with bleeding disorders in the Philippines. Reprinted with permission from Jojo Silvestre, the Daily Tribune. https://tribune.net.ph/index.php/2019/04/30/ambassador-for-bleeding-disorders/

Star was born with bruises all over her body. At three weeks old, she had a nosebleed that prompted her parents to take her to the emergency room.

Andrea told the doctors that her family has a history of bleeding on both sides. A nephew from her father’s side just got diagnosed with severe hemophilia.

“‘No, she can’t have hemophilia. She is a girl,’ the doctors told me,” recounts Andrea.
Star grew up having at least one nosebleed episode every week. On bad days, she would nosebleed daily. Finally, in 2006, Andrea brought Star to Hong Kong. It was there that she and Star were finally diagnosed with a bleeding disorder called Von Willebrand Disease (VWD).

More common than hemophilia

Hemophilia, VWD and other inherited bleeding disorders have similar symptoms — bruising, nosebleeds, gum bleeds, excessive periods, to name a few. While hemophilia is the most known bleeding disorder, VWD is actually the most common. According to the WFH, VWD affects roughly 1 to 2 percent of the population.

“That was also when I realized that there are very limited resources here for people with bleeding disorders,” Andrea shares. “Managing bleeding disorders is expensive. But the scarcity of medicines — the factors — is something that truly surprised me. That even if you have the capacity to purchase these medicines, there are times you can’t find enough supply.”

Andrea soon started a blog to share the family’s experiences and in some ways, reach out to people around the world who have the same bleeding disorder. She was surprised by the amount of attention her blog received and the number of people who linked up with her. She then saw an opportunity to learn more about rare bleeding disorders and share with others whatever knowledge she could gather. It opened a whole new world for her.

Founding Hemophilia Advocates

In 2008, Andrea met Laurie Kelley, founder of two humanitarian programs — Save One Life and Project SHARE. Laurie came to the Philippines to do a fact-finding mission to gather data on the situation of persons with hemophilia in the Philippines. She invited Andrea to come along. It was an eye-opener to the very sad situation of Filipinos with bleeding disorders. She vowed to help the community and became a local volunteer for Project SHARE and Save One Life.

In 2011, she was invited to become an ambassador for My Girls Blood, a US-based non profit specifically aimed at raising awareness that women can also be affected with bleeding disorders.

In 2016, Andrea along with four others, founded Hemophilia Advocates-Philippines (HAP). The group’s main goal is to push for government-funded treatment for people affected by bleeding disorders.

“Hemophilia and bleeding disorders are lifelong but manageable. If we have access to treatment, we can lead normal lives,” she says.

But that is not so for most affected Filipinos. Treatment for a simple, non-life-threatening bleed, such as internal bleed in the joints, could cost around P100,000 for a single infusion for an adult.

Through the network Andrea helped to build among donors and beneficiaries, HAP has been able to bridge that gap. International organizations, such as Project SHARE, send medicines to HAP, which in turn, distribute to those who badly need them.

Global ambassador

Andrea’s personal advocacy has brought her to different parts of the globe, at her own expense, to link up with other organizations, so she could learn more about it and also seek help for the less privileged Filipinos who also have the bleeding disorder but don’t have the means to get treatment.

“Being an advocate has helped me more,” Andrea says. “Our journey with von Willebrand Disease is no longer as lonely as it used to be. We now belong to a real community of people who ‘speak the same language.’”

When Facebook introduced the Facebook Group, Andrea started a support group, which then became the HAP, which celebrated its second anniversary recently. Aside from providing factors, the organization also helps empower patients through scholarships and business opportunities.

Legislative advocate

Since she started the blog, Andrea worked nonstop with her advocacy. Today, she is busy lobbying for the passage of Senate Bill 1335 or the “Bleeding Disorders Standard of Care Bill,” which seeks to give free treatment for persons with bleeding disorders such as hemophilia, VWD and other inherited bleeding disorders. The bill also seeks the establishment of Hemophilia Treatment Centers across the country.

She also hopes that through the proposed bill, medicines, especially donated factors from overseas, will reach its recipients the soonest possible time.

Andrea and her daughter still occasionally need blood transfusions but she no longer feels helpless unlike before. She is able to live with the disorder while seeking help for others at the same time.

“If I wanted to, I can just focus all my energy on me and my daughter,” she says. “But I believe that I inherited this disorder for a reason. For as long as I can, and for as long as the hemo community needs me, I will continue with my advocacy of helping them.”

Know Your Factor Choices

It’s truly a new era in bleeding disorders with new products, new therapies and new indications. Octapharma’s wilate just got approval for indication in adults and adolescents with hemophilia A; previously it was indicated only for VWD. With this news, I realized that we should remind everyone to know what product they use, where it comes from, and why they are using it.

Who chose your product? Was it your hematologist? Did you have any input? You should, and this means bearing the responsibility to read about products, as questions about them and be part of the decision making process.

Back in 1990, when Raising a Child with Hemophilia was first published, we were the first ones to describe parents and patients as consumers, and to treat products like any other commercial product you would buy. We provided the question to ask your doctor, and we explained the different manufacturing processes, and the difference between safety and purity. And we urged parents not to be passive in the treatment process but to help make medical decisions.

It’s more important now than ever. We have a dazzling choice of products, and novel therapies like Hemlibra. Maybe there’s no reason for you to switch products, but you might want to get up to speed on what’s available. Please note: all US FDA-approved products are considered safe and efficacious.

We’ve been writing about products and product choice for decades. I hope you read up on them and be prepared! To help you, we created a chart of products that you can download. Take it to the HTC with you and discuss with your hematologist about choice. You can download it right from our home page or go here.

We also have a chart for VWD products too!

We did our part. Now do yours; know your product, manufacturer, and why you use it. Keep learning… more therapy choices are coming!

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